Blastic Plasmacytoid Dendritic Cell Neoplasm With Central Nervous System Involvement: A Case Report

Blastic plasmacytoid dendritic cell neoplasm is a rare hematologic neoplasm characterized by cutaneous, hematologic, and central nervous system (CNS) involvement with poor prognosis. Diagnosis is made by flow cytometry, although there are no specific markers, making its diagnosis challenging. So far, with the available evidence, acute lymphoid leukemia-type schemes and consolidation with allogeneic transplant seem to become the first-line therapy. With its characterization, new therapies directed toward CD 123 and the anti-apoptotic protein Bcl-2 have appeared to prolong the survival of these patients. We present a case of a 27-year-old male patient diagnosed with blastic plasmacytoid dendritic cell neoplasm with unusual CNS manifestations and without skin involvement who achieved complete remission with venetoclax and improvement of neurological symptoms, making him a candidate for hematopoietic stem cell transplant.

Automated summary

Blastic plasmacytoid dendritic cell neoplasm is a rare hematologic neoplasm with cutaneous, hematologic, and central nervous system involvement. Diagnosis is challenging, and the first-line therapy includes acute lymphoid leukemia-type schemes and allogeneic transplant. New therapies targeting CD 123 and Bcl-2 have shown promise in improving patient survival.